Back to Search
Start Over
Neuroimaging of Dandy-Walker malformation: new concepts.
- Source :
-
Topics in magnetic resonance imaging : TMRI [Top Magn Reson Imaging] 2011 Dec; Vol. 22 (6), pp. 303-12. - Publication Year :
- 2011
-
Abstract
- Dandy-Walker malformation (DWM) is the most common human cerebellar malformation, characterized by hypoplasia of the cerebellar vermis, cystic dilation of the fourth ventricle, and an enlarged posterior fossa with upward displacement of the lateral sinuses, tentorium, and torcular. Although its pathogenesis is not completely understood, there are several genetic loci related to DWM as well as syndromic malformations and congenital infections. Dandy-Walker malformation is associated with other central nervous system abnormalities, including dysgenesis of corpus callosum, ectopic brain tissue, holoprosencephaly, and neural tube defects. Hydrocephalus plays an important role in the development of symptoms and neurological outcome in patients with DWM, and the aim of surgical treatment is usually the control of hydrocephalus and the posterior fossa cyst. Imaging modalities, especially magnetic resonance imaging, are crucial for the diagnosis of DWM and distinguishing this disorder from other cystic posterior fossa lesions. Persistent Blake's cyst is seen as a retrocerebellar fluid collection with cerebrospinal fluid signal intensity and a median line communication with the fourth ventricle, commonly associated with hydrocephalus. Mega cisterna magna presents as an extraaxial fluid collection posteroinferior to an intact cerebellum. Retrocerebellar arachnoid cysts frequently compress the cerebellar hemispheres and the fourth ventricle. Patients with DWM show an enlarged posterior fossa filled with a cystic structure that communicates freely with the fourth ventricle and hypoplastic vermis. Comprehension of hindbrain embryology is of utmost importance for understanding the cerebellar malformations, including DWM, and other related entities.
- Subjects :
- Abnormalities, Multiple genetics
Cerebellum abnormalities
Cranial Fossa, Posterior parasitology
Dandy-Walker Syndrome genetics
Female
Fetal Diseases diagnosis
Genetic Counseling
Humans
Hydrocephalus diagnosis
Infant, Newborn
Male
Pregnancy
Rhombencephalon abnormalities
Abnormalities, Multiple diagnosis
Cranial Fossa, Posterior abnormalities
Dandy-Walker Syndrome diagnosis
Magnetic Resonance Imaging methods
Neuroimaging methods
Prenatal Diagnosis methods
Subjects
Details
- Language :
- English
- ISSN :
- 1536-1004
- Volume :
- 22
- Issue :
- 6
- Database :
- MEDLINE
- Journal :
- Topics in magnetic resonance imaging : TMRI
- Publication Type :
- Academic Journal
- Accession number :
- 24132069
- Full Text :
- https://doi.org/10.1097/RMR.0b013e3182a2ca77